皮膚科 劉權毅醫師

lupus pernios: a variant of sarcoidosis
chilblain lupus: a variant of CLE

Like lupus pernio, CL is characterized by erythematosus-purple plaques located in acral areas (most often, the nose and ears) but that are induced by exposure to cold or a drop in temperature (Fig 1), unlike other lesions of lupus erythematosus, which worsen with sun exposure, and sarcoidosis lesions, which remain unaltered with changes in temperature. CL can cause mild pain or itching, and can be associated with hyperhidrosis. During the evolution of the disease, the presence of atrophic scarring and residual pigmentation are common findings. Like others forms of lupus lesions, this is more common in women, and although it is sporadic, two families with autosomal-dominant inherited CL have been reported.

The main difference with the cutaneous variety of sarcoidosis is the histopathologic study findings. In patients with lupus pernio, we observe granulomas without caseous necrosis but with few inflammatory infiltrates on the periphery; however, in patients with CL epidermal atrophy, the degeneration of the basal layer, periadnexal and perivascular inflammatory infiltrates are found, and other uncommon findings, such as dyskeratosis, increased mucin in the dermis, and the presence of granular deposits of Igs and complement in basement membrane, have been described.

To establish a proper diagnosis, Su et al[3] suggested using the Mayo Clinic diagnostic criteria. These comprise two major criteria (skin lesions in acral locations induced by exposure to cold or a drop in temperature and evidence of lupus erythematosus in the skin lesions, as determined by histopathologic examination or indirect immunofluorescence study) and four minor criteria (coexistence of systemic lupus erythematosus or other skin lesion of discoid lupus erythematosus, response to anti-lupus therapy, and negative results of cryoglobulin and cold agglutinin studies). Both major criteria and one minor criterion need to be present to diagnose CL.

Recently, a treatment review of CL was conducted[4] that stressed the importance of protection from cold by physical measures as a trigger factor and the use of topical or oral antibiotics if the lesions are infected. Therapy with topical corticosteroids or calcium channel blockers get a good response from patients. In patients with severe cases, systemic corticosteroids and mycophenolate may be used. However, according to these authors,[4] therapy with antimalarial agents has a minor effect. Conversely, therapy with infliximab appears to be superior to therapy with systemic corticosteroids, with or without additional agents, for the treatment of lupus pernio.[1] Instead, infliximab does not seem useful in the treatment of CL; even a case of CL induced by this drug has been described.[5] In any case, it is essential to make a correct diagnosis; although clinically the two entities are similar and have in common their association with systemic disease (lupus erythematosus or sarcoidosis), the prognosis and treatment differ considerably.

參考文獻
Chest. 2009 Sep;136(3):946-7; author reply 947.
Lupus pernio or chilblain lupus?: two different entities
http://www.mdconsult.com/das/article/body/238742652-2/jorg=journal&source=&sp=22504055&sid=0/N/721502/1.html?issn=0012-3692

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